Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Aristotle University of Thessaloniki, 49 Konstantinoupoleos Str, 54642, Thessaloniki, Greece.
Arthritis Research UK Centre for Epidemiology, University of Manchester, Oxford Road, Manchester, M13 9PT, UK.
Rheumatol Int. 2018 Aug;38(8):1345-1354. doi: 10.1007/s00296-018-4074-5. Epub 2018 Jun 4.
Inflammasomes are large intracellular complexes that induce inflammation in response to exogenous and endogenous damage signals. They regulate production and release of the proinflammatory cytokines IL-1β and IL-18, playing a defensive role against infections. Inflammasomes have also been involved in the pathogenesis of a wide range of autoinflammatory conditions that are caused by dysregulation of the IL-1 pathway, such as cryopyrinopathies and hereditary periodic fever syndromes. On top of that, research in recent years suggests that defects in inflammasome regulation and signaling associate with a number of autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and others. In this review, we describe the inflammasome and mechanisms that trigger it, provide a brief review of autoinflammatory disorders and discuss the current understanding and emerging data from experimental and clinical studies for the role of the innate immune system and inflammasomes in the biology and pathogenesis of systemic autoimmune diseases.
炎症小体是一种大型的细胞内复合物,可对外源和内源性损伤信号作出反应引发炎症。它们调控促炎细胞因子白细胞介素-1β(IL-1β)和白细胞介素-18(IL-18)的产生和释放,在对抗感染方面发挥防御作用。炎症小体还参与了广泛的自身炎症性疾病的发病机制,这些疾病是由 IL-1 途径的失调引起的,如 Cryopyrin 相关周期性综合征和遗传性周期性发热综合征。除此之外,近年来的研究表明,炎症小体调节和信号转导的缺陷与许多自身免疫性疾病有关,如类风湿关节炎、系统性红斑狼疮、系统性硬皮病等。在这篇综述中,我们描述了炎症小体及其触发机制,简要回顾了自身炎症性疾病,并讨论了目前对固有免疫系统和炎症小体在系统性自身免疫性疾病的生物学和发病机制中的作用的理解和来自实验及临床研究的新数据。